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Autors principals: Anna Seng, Pareena Sharma, Kareem Wali, Khoa Tran, Charline Boente, Chris Ashton, Asim Ali, Gregory Borschel
Format: Recurso digital
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Publicat: Zenodo 2026
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Accés en línia:https://doi.org/10.5281/zenodo.20100081
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  • PURPOSE: Pontine tegmental cap dysplasia (PTCD) is a rare congenital hindbrain malformation associated with multiple cranial neuropathies, often causing facial palsy, sensorineural hearing loss, and neurotrophic keratopathy (NK). Effective treatment options for NK in patients with PTCD are limited. And, corneal neurotization (CN) represents the only surgical intervention that addresses corneal denervation in NK. The purpose of this study is to report and contextualize the surgical approaches and outcomes of CN in patients with PTCD who developed NK. METHODS: A cohort of 13 patients with congenital NK secondary to PTCD was identified among 56 patients who underwent CN at two institutions. Data was shared between two centers using REDCap. Perioperative course, CN surgical techniques, and postoperative outcomes, including ocular surface integrity and corneal sensitivity, were analyzed and qualitatively described. RESULTS: All patients presented with Mackie stage 2-3 NK. Prior interventions included lubrication, bandage contact lenses, artificial tears, and antibiotics; one patient also received cenegermin. Medical management yielded minimal benefit, and all patients subsequently underwent CN. Donor nerve viability was determined by dermatome pinch testing pre-operatively. The supratrochlear nerve was used in three cases, and the greater auricular nerve in eleven. By postoperative month 12, all patients showed ocular surface protection and improved corneal integrity. CONCLUSION: CN demonstrated clear benefit in preserving corneal integrity in PTCD-associated NK, with gradual improvements in sensitivity. The greater auricular nerve, though unconventional, proved a practical donor option in this cohort. CN should be considered a valuable therapeutic option for this complex population. *Source: https://ps-rc.org/meeting/Program/2026/OS79.cgi*